Background: Dermatomyositis (DM) is a multisystem idiopathic inflammatory disorder that most commonly affects the muscles and skin. Systemic corticosteroids are the mainstay of therapy but are limited by their longterm adverse effects.Observations: We sought to evaluate the effectiveness of oral mycophenolate mofetil in patients with cutaneous lesions of DM recalcitrant to other therapies through an open-label retrospective medical chart review of patients in a university-affiliated private practice setting. Twelve patients with DM who had skin lesions recalcitrant to traditional therapies or who developed toxic effects from traditional therapies began mycophenolate mofetil treatment at doses ranging from 500 mg to 1 g twice a day. Response was based on improvement in skin disease as judged clinically, an increase in strength, and/or an ability to decrease or discontinue concomitant therapies. Improvement was seen in 10 of the 12 patients, most within 4 to 8 weeks. Most patients tolerated mycophenolate mofetil treatment without problem; however, 1 patient developed a B-cell lymphoma of the central nervous system, and another developed abnormal levels of hepatic enzymes along with urinary symptoms. Resolution of these toxic reactions occurred with cessation of mycophenolate mofetil treatment in each patient.
Conclusion:Mycophenolate mofetil may be an effective corticosteroid-sparing therapy for the treatment of some patients with DM.
Multicentric reticulohistiocytosis is a rare multisystem disorder in which an infiltration of histiocytic cells causes papulonodular skin lesions and potentially a destructive polyarthritis. The active disease typically resolves spontaneously after 5-8 years, but the articular destruction can lead to permanent joint deformities. We present a case of multicentric reticulohistiocytosis in a 14-year-old girl. The number of papules on her hands decreased in number and her arthritic symptoms improved after 4 months of oral naproxen. Thirty months later her joint symptoms remained inactive and only six very small papules remained on her hands. Small flexor deformities were present in the distal interphalangeal joints of both index fingers. This case is an example of how multicentric reticulohistiocytosis can be a relatively stable and self-limited disease but still cause permanent joint deformities.
There is a group of patients that mirror the characteristics of our animal model. Accordingly, at least one etiology of preeclampsia is related to excessive expansion of the extracellular fluid volume.
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